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MCID: OSC001
MIFTS: 32

Oeis Complex

Categories: Fetal diseases, Nephrological diseases, Rare diseases
Genes Tissues Related diseases Publications Symptoms & Phenotypes
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Aliases & Classifications for Oeis Complex

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MalaCards integrated aliases for Oeis Complex:

Name: Oeis Complex 57 59
Omphalocele-Cloacal Exstrophy-Imperforate Anus-Spinal Defect Syndrome 59
Omphalocele-Exstrophy-Imperforate Anus-Spinal Defects 57
Omphalocele Exstrophy Imperforate Anus 73
Cloacal Exstrophy 59

Characteristics:

Orphanet epidemiological data:

59
cloacal exstrophy
Inheritance: Multigenic/multifactorial; Age of onset: Infancy,Neonatal; Age of death: normal life expectancy;

OMIM:

57
Miscellaneous:
majority of cases are sporadic
oeis is an acronym for omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects

Inheritance:
isolated cases


HPO:

32
oeis complex:
Inheritance sporadic


Classifications:

MalaCards categories:
Global: Fetal diseases Rare diseases
Anatomical: Nephrological diseases
See all MalaCards categories (disease lists)
ICD10: 34
Orphanet: 59  
Rare renal diseases
Developmental anomalies during embryogenesis


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Summaries for Oeis Complex

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MalaCards based summary : Oeis Complex, also known as omphalocele-cloacal exstrophy-imperforate anus-spinal defect syndrome, is related to bladder exstrophy-epispadias-cloacal exstrophy complex and fronto nasal malformation cloacal exstrophy. An important gene associated with Oeis Complex is MNX1 (Motor Neuron And Pancreas Homeobox 1). Affiliated tissues include kidney, colon and uterus, and related phenotypes are talipes equinovarus and anal atresia

Wikipedia : 76 Cloacal exstrophy (EC) is a severe birth defect wherein much of the abdominal organs (the bladder and... more...

Description from OMIM: 258040
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Related Diseases for Oeis Complex

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Diseases related to Oeis Complex via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 34)
# Related Disease Score Top Affiliating Genes
1 bladder exstrophy-epispadias-cloacal exstrophy complex 12.7
2 fronto nasal malformation cloacal exstrophy 12.3
3 omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects complex 12.0
4 cloacal exstrophy 11.5
5 omphalocele 10.6
6 anus, imperforate 10.6
7 epispadias 10.4
8 gastroschisis 10.3
9 compartment syndrome 10.3
10 abdominal wall defect 10.3
11 myelocystocele 10.3
12 pentalogy of cantrell 10.1
13 congenital heart defects, hamartomas of tongue, and polysyndactyly 10.1
14 diastematomyelia 10.1
15 duodenal atresia 10.1
16 deafness, aminoglycoside-induced 10.1
17 tethered spinal cord syndrome 10.1
18 squamous cell carcinoma 10.1
19 teratoma 10.1
20 ovarian cyst 10.1
21 mature teratoma 10.1
22 intestinal obstruction 10.1
23 46, xy disorders of sexual development 10.1
24 arachnoid cysts 10.1
25 sirenomelia 10.1
26 syringohydromyelia 10.1
27 penile agenesis 10.1
28 nail-patella syndrome 10.0
29 neural tube defects 10.0
30 exstrophy of bladder 10.0
31 kidney disease 10.0
32 double discordia 10.0
33 limb-body wall complex 10.0
34 transposition of the great arteries 10.0

Graphical network of the top 20 diseases related to Oeis Complex:



Diseases related to Oeis Complex
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Symptoms & Phenotypes for Oeis Complex

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Symptoms via clinical synopsis from OMIM:

57
Neurologic Central Nervous System:
hydrocephalus
tethered cord
meningomyelocele
lipomyelocystoceles
chiari malformation

Skeletal Pelvis:
congenital hip dislocation
symphysis pubis diastasis

Genitourinary Kidneys:
pelvic kidney
hydronephrosis
renal agenesis

Genitourinary External Genitalia Male:
ambiguous genitalia
epispadias
small penis
abnormal or absent scrotum

Genitourinary Ureters:
hydroureter
duplicated collecting system

Chest Ribs Sternum Clavicles And Scapulae:
11 pairs of ribs

Genitourinary Bladder:
cloacal exstrophy
vesicovaginal fistula

Genitourinary Internal Genitalia Male:
cryptorchidism

Abdomen Gastrointestinal:
anal atresia
intestinal malrotation
rectovaginal fistula
anteriorly placed anus
duplicated colon
more
Skeletal Spine:
hemivertebrae
absent sacrum
sacral segmentation defects

Genitourinary External Genitalia Female:
ambiguous genitalia
hypoplastic labia
widely separated labioscrotal folds

Abdomen External Features:
omphalocele

Genitourinary Internal Genitalia Female:
bifid uterus

Skeletal Feet:
club feet


Clinical features from OMIM:

258040

Human phenotypes related to Oeis Complex:

59 32 (show all 47)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 talipes equinovarus 59 32 Frequent (79-30%) HP:0001762
2 anal atresia 59 32 Frequent (79-30%) HP:0002023
3 intestinal malrotation 59 32 Frequent (79-30%) HP:0002566
4 hemivertebrae 59 32 Frequent (79-30%) HP:0002937
5 hydroureter 59 32 Occasional (29-5%) HP:0000072
6 omphalocele 59 32 Very frequent (99-80%) HP:0001539
7 myelomeningocele 59 32 Frequent (79-30%) HP:0002475
8 bladder exstrophy 59 32 Very frequent (99-80%) HP:0002836
9 cloacal exstrophy 59 32 Very frequent (99-80%) HP:0010475
10 hydrocephalus 32 HP:0000238
11 ectopic kidney 59 Occasional (29-5%)
12 cryptorchidism 32 HP:0000028
13 horseshoe kidney 59 Occasional (29-5%)
14 renal hypoplasia/aplasia 59 Occasional (29-5%)
15 congenital hip dislocation 32 HP:0001374
16 hip dislocation 59 Frequent (79-30%)
17 arnold-chiari malformation 32 HP:0002308
18 vesicoureteral reflux 59 Very frequent (99-80%)
19 spina bifida 59 Frequent (79-30%)
20 hypoplasia of penis 59 Very frequent (99-80%)
21 pelvic kidney 32 HP:0000125
22 rectovaginal fistula 32 HP:0000143
23 anteriorly placed anus 32 HP:0001545
24 ureteropelvic junction obstruction 59 Occasional (29-5%)
25 hydronephrosis 32 HP:0000126
26 epispadias 32 HP:0000039
27 micropenis 32 HP:0000054
28 ambiguous genitalia, male 32 HP:0000033
29 abnormality of tibia morphology 59 Frequent (79-30%)
30 renal agenesis 32 HP:0000104
31 absence of the sacrum 32 HP:0010305
32 11 pairs of ribs 32 HP:0000878
33 abnormality of the fibula 59 Frequent (79-30%)
34 labial hypoplasia 32 HP:0000066
35 intestinal duplication 59 Frequent (79-30%)
36 ambiguous genitalia, female 32 HP:0000061
37 bifid uterus 32 HP:0000136
38 ureterocele 59 Occasional (29-5%)
39 duplicated collecting system 32 HP:0000081
40 abnormality of the fallopian tube 59 Occasional (29-5%)
41 abnormality of the clitoris 59 Very frequent (99-80%)
42 duplicated colon 32 HP:0005223
43 absent foot 59 Frequent (79-30%)
44 tethered cord 32 HP:0002144
45 vesicovaginal fistula 32 HP:0001586
46 absent scrotum 32 HP:0008707
47 sacral segmentation defect 32 HP:0008490
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Drugs & Therapeutics for Oeis Complex

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Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Prospective Cohort of Transitional Urology Patients Recruiting NCT03061084

Search NIH Clinical Center for Oeis Complex

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Genetic Tests for Oeis Complex

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Anatomical Context for Oeis Complex

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MalaCards organs/tissues related to Oeis Complex:

41
Kidney, Colon, Uterus
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Publications for Oeis Complex

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Articles related to Oeis Complex:

(show all 41)
# Title Authors Year
1
A rare case of OEIS complex - newer approach to diagnosis of exstrophy bladder by color doppler and its differentiation from simple omphalocele. ( 29379239 )
2017
2
Discordant Anomalies with Combined Features of Pentalogy of Cantrell and OEIS Complex: A Case Report in Monochorionic Twins. ( 28617177 )
2017
3
Penile duplication as genital abnormality in OEIS complex: a fetal autopsy case report. ( 26513516 )
2016
4
Multimodality Renal Failure in a Patient with OEIS Complex. ( 26495176 )
2015
5
Fetal and neonatal presentation of OEIS complex. ( 26520699 )
2015
6
Early prenatal diagnosis of the OEIS complex with different appearance in early compared with late pregnancy before spontaneous rupture of the cloacal membrane. ( 24623061 )
2014
7
Re: Urological outcomes in the omphalocele exstrophy imperforate anus spinal defects (OEIS) complex: experience with 80 patients. ( 24703152 )
2014
8
Omphalocele, exstrophy of cloaca, imperforate anus and spinal defect (OEIS Complex) with overlapping features of body stalk anomaly (limb body wall complex). ( 25400352 )
2014
9
Urological outcomes in the omphalocele exstrophy imperforate anus spinal defects (OEIS) complex: experience with 80 patients. ( 22640865 )
2013
10
OEIS complex. ( 24839537 )
2013
11
Prenatal findings of omphalocele-exstrophy of the bladder-imperforate anus-spinal defects (OEIS) complex. ( 22925220 )
2012
12
Gastrointestinal reconstruction and outcomes for patients with the OEIS complex. ( 21453858 )
2011
13
Comprehensive genetic analysis of OEIS complex reveals no evidence for a recurrent microdeletion or duplication. ( 21204209 )
2011
14
Prenatal diagnosis of OEIS (omphalocele, bladder exstrophy, imperforate anus, clubfeet) variant associated with increased nuchal translucency and OEIS complex with ambiguous genitalia associated with corrected transposition of the great arteries: case series and review of the literature. ( 21475965 )
2011
15
Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex in a child with nail-patella syndrome. ( 20880310 )
2010
16
OEIS complex associated with chromosome 1p36 deletion: a case report and review. ( 20101692 )
2010
17
Supernumerary kidney in a child with OEIS complex. ( 19371928 )
2009
18
Discordant omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex and cardiac malformations in monochorionic twins. ( 19842883 )
2009
19
Double lumbosacral lipomas of the dorsal and filar types associated with OEIS complex: case report. ( 19855150 )
2009
20
Neurosurgical management of occult spinal dysraphism associated with OEIS complex. ( 17962955 )
2008
21
Syndromes and disorders associated with omphalocele (II): OEIS complex and Pentalogy of Cantrell. ( 17638617 )
2007
22
Prenatal ascertainment of OEIS complex/cloacal exstrophy - 15 new cases and literature review. ( 17702047 )
2007
23
OEIS complex with major cardiac malformation: a case report. ( 17883075 )
2007
24
OEIS complex: prenatal ultrasound and autopsy findings. ( 17221944 )
2007
25
OEIS complex with glomerulocystic kidney disease: a case report. ( 17378687 )
2007
26
The OEIS complex: two case reports that illustrate the spectrum of abnormalities and a review of the literature. ( 16450352 )
2006
27
OEIS complex with del(3)(q12.2q13.2). ( 15887303 )
2005
28
Anogenital malformation with ambiguous genitalia as part of the OEIS complex. ( 15543545 )
2004
29
Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex associated with increased nuchal translucency. ( 12858313 )
2003
30
OEIS complex, VATER, and the ongoing difficulties in terminology and delineation. ( 11807874 )
2002
31
OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects): a review of 14 cases. ( 11251992 )
2001
32
Exstrophy of the cloaca and the OEIS complex: one and the same. ( 11251991 )
2001
33
OEIS complex--a population study. ( 10797425 )
2000
34
OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects) in monozygotic twins. ( 10213043 )
1999
35
Perinatal features of omphalocele-exstrophy-imperforate anus-spinal defects (OEIS complex) associated with large meningomyeloceles and severe limb defects. ( 9259943 )
1997
36
Severe cardiac defect in a patient with the OEIS complex. ( 9354848 )
1997
37
Omphalocele-exstrophy-imperforate-anus-spina bifida (OEIS) complex in a male prenatally exposed to diazepam. ( 7711456 )
1995
38
OEIS complex with craniofacial anomalies--defect of blastogenesis? ( 7802030 )
1994
39
The OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects): recurrence in sibs. ( 1433234 )
1992
40
OEIS complex (cloacal exstrophy): prenatal diagnosis in the second trimester. ( 2456564 )
1988
41
The OEIS complex (omphalocele, exstrophy, imperforate anus, spinal defects). ( 728566 )
1978
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Variations for Oeis Complex

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Expression for Oeis Complex

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Search GEO for disease gene expression data for Oeis Complex.
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Pathways for Oeis Complex

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GO Terms for Oeis Complex

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Sources for Oeis Complex

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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